Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

Autor: Robert P. Baughman, Steven D. Nathan, Joseph Barney, Francis Cordova, Daniel A. Culver, Vasilis Kouranos, Joseph G Cal, Elyse E. Lower, Mary Beth Scholand, JoAnne Baran, Marlies S. Wijsenbeek, Oksana A. Shlobin, Sivagini Ganesh, Surinder S. Birring, Athol U. Wells, Peter J. Engel, Eva M. Carmona, Esam H. Alhamad, Lanier O'Hare
Přispěvatelé: Pulmonary Medicine
Rok vydání: 2018
Předmět:
Zdroj: Respiratory Medicine, 139, 72-78. W.B. Saunders
Baughman, R P, Shlobin, O A, Wells, A U, Alhamad, E H, Culver, D A, Barney, J, Cordova, F C, Carmona, E M, Scholand, M B, Wijsenbeek, M, Ganesh, S, Birring, S S, Kouranos, V, O'Hare, L, Baran, J M, Cal, J G, Lower, E E, Engel, P J & Nathan, S D 2018, ' Clinical features of sarcoidosis associated pulmonary hypertension : Results of a multi-national registry ', Respiratory Medicine, pp. 72-78 . https://doi.org/10.1016/j.rmed.2018.04.015
ISSN: 0954-6111
Popis: Background Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. Methods Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD). Results A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = −0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = −0.362, p Conclusions The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.
Databáze: OpenAIRE